Outcome of the Lima periurethral constrictor in children and adolescents: a European perspective.

PURPOSE: The Lima constrictor was described in 1996 as a less complex and less expensive alternative to the artificial urinary sphincter for use in cases of pediatric neuropathic sphincter incontinence. The device provides a fixed periurethral resistance which creates continence, yet allows urethral catheterization without the need to deflate the cuff. We report our multicenter experience, and continence, revision and erosion rates. MATERIALS AND METHODS: We performed a retrospective review of 14 consecutive patients who underwent insertion of the periurethral constrictor (Silimed, Rio de Janeiro, Brazil) between 2005 and 2011. Data are presented as medians (range). RESULTS: A total of 14 patients (13 male, 1 female) with spina bifida (10), sacral agenesis (3) and Hirschsprung disease (1) underwent insertion of the constrictor at a median age of 12 years (range 8 to 20). All patients were wet despite clean intermittent catheterization, medical therapy and/or previous surgery. Eleven patients underwent simultaneous bladder augmentation and/or Mitrofanoff formation. The constrictor was activated a median of 8 weeks (range 2 to 99) after the procedure in 11 patients whereas 3 became dry without activation. Complications occurred in 4 patients (29%), including spontaneous bladder perforation and constrictor erosion (1), tubing disconnection requiring revision (2) and wound infection (1). At a median of 23 months of followup (range 7 to 77) 13 patients were dry and 1 was damp. All patients performed urethral or Mitrofanoff clean intermittent catheterization. The continence rate with the device in situ was 92%. CONCLUSIONS: At a median followup of 23 months the Lima constrictor provided a 92% continence rate with erosion and revision rates of 7% and 14%, respectively. Interim results suggest that the constrictor provides a safe and effective surgical option, particularly in patients who are unable to void to completion.

The biomechanical and functional relationships of the proximal radioulnar joint, distal radioulnar joint, and interosseous ligament.

This biomechanical study assessed integrated function of the proximal radioulnar joint (PRUJ), interosseous ligament (IOL), and distal radioulnar joint (DRUJ). Tekscan™ pressure sensors were inserted into the DRUJ and PRUJ of 15 cadaveric specimens. MicroStrain(®) sensors were mounted onto the IOL on nine of these specimens. A customized biomechanical jig was used to apply axial loads and take measurements through pronosupination. The PRUJ, IOL, and DRUJ were shown to function as an integrated osseoligamentous system distributing applied load. The PRUJ has transmitted pressure profiles similar to those of the DRUJ. Different IOL components support loading at different stages of pronosupination. The IOL is lax during pronation. Mid-IOL tension peaks in the midrange of forearm rotation; distal-IOL tension peaks in supination. Axial loading consistently increases IOL strain in a non-linear fashion. There are clinical implications of this work: disease or surgical modification of any of these structures may compromise normal biomechanics and function.

The correspondence bias.

The correspondence bias is the tendency to draw inferences about a person's unique and enduring dispositions from behaviors that can be entirely explained by the situations in which they occur. Although this tendency is one of the most fundamental phenomena in social psychology, its causes and consequences remain poorly understood. This article sketches an intellectual history of the correspondence bias as an evolving problem in social psychology, describes 4 mechanisms (lack of awareness, unrealistic expectations, inflated categorizations, and incomplete corrections) that produce distinct forms of correspondence bias, and discusses how the consequences of correspondence-biased inferences may perpetuate such inferences.

Clonal chromosomal aberrations in a leiomyosarcoma of the sinonasal tract.

We report the first cytogenetic analysis of a leiomyosarcoma of the sinonasal tract, a rare neoplasm. Karyotypic analysis showed near-triploid and near-tetraploid modal chromosome numbers with extensive structural and numerical aberrations. Three consistent structural changes, including i(6p), der(10)ins(10;1)(q26;q23q44), and der(12)t(1;12)(q11;q24) were observed in most cells. A der(11)t(11;?)(p15;?) was observed in 14 of 20 cells. Clonal structural rearrangements, including i(1q), del(2)(q37), der(3)t(3;?)(p25;?), del(4)(q31), del(7)(q32), der(12)t(12;?)(p12;?), der(15), del(21)(q22), and der(X) were each observed in a few cells. Numerical changes, including trisomies for chromosomes 2-5, 7, 9, 11, 15, 17, 18, and 20 and monosomies 10 and 12 were observed. Comparison of our findings to those of leiomyosarcomas at different sites showed trisomies 7 and 20 and rearrangements of 11p12-p15 and 21q22.

Consistent numerical chromosome aberrations in congenital fibrosarcoma.

Cytogenetic analysis of a congenital fibrosarcoma of the volar forearm from a 2.5-month-old boy revealed a mosaic karyotype 46,XY/49,XY,+11,+17,+20. This pattern of specific trisomies provides additional support to the cytogenetic findings in five cases of congenital fibrosarcoma reported previously. Trisomy 11 appears to be characteristic of congenital fibrosarcoma with additional trisomies 8, 17, and 20 as common findings.

You can't not believe everything you read.

Can people comprehend assertions without believing them? Descartes (1644/1984) suggested that people can and should, whereas Spinoza (1677/1982) suggested that people should but cannot. Three experiments support the hypothesis that comprehension includes an initial belief in the information comprehended. Ss were exposed to false information about a criminal defendant (Experiments 1 and 2) or a college student (Experiment 3). Some Ss were exposed to this information while under load (Experiments 1 and 2) or time pressure (Experiment 3). Ss made judgments about the target (sentencing decisions or liking judgments). Both load and time pressure caused Ss to believe the false information and to use it in making consequential decisions about the target. In Spinozan terms, both manipulations prevented Ss from "unbelieving" the false information they automatically believed during comprehension.

The Malone antegrade continence enema.

The previously reported Malone antegrade continence enema (MACE) was used in 21 children for intractable fecal incontinence or constipation. Twelve are completely clean, and three are much improved (71% success rate). However, only four of the 21 have not had some type of complication, either minor or major. Five of the 21 now have a colostomy, and one has abandoned the procedure.

Aberrant thymus: a misleading mediastinal mass.

We report two cases in which aberrant thymic tissue caused a misleading superior mediastinal mass. The diagnosis can be made by awareness of it and by use of appropriate investigations. Should surgery be performed we recommend frozen section biopsy if ectopic thymus is suspected, and if confirmed, the mass should not be excised unless it is causing serious mechanical problems.

The MACE procedure: experience in the United Kingdom.

BACKGROUND/PURPOSE: The Malone Antegrade Continence enema (MACE) procedure has become commonplace in the treatment of patients with faecal incontinence and constipation. To ascertain its place in paediatric practice, the authors surveyed members of the British Association of Paediatric Surgeons (BAPS) to see if they have performed a MACE and continue to do so. The authors also assessed their indications, success rate, and complications. METHODS: Members were asked to give the diagnosis of the patients submitted for surgery before September 1996, the procedure used to constructthe MACE, the rate of failure, and complications encountered. RESULTS: A total of 300 MACE were reported, and the mean follow-up was 2.4 years. The diagnosis of 273 patients was known: spina bifida, 108; anorectal anomaly, 90; Hirschsprung's disease, 22; constipation, 23; other, 30. The most popular procedure was appendix disconnection and reimplantation. The overall success rate for all diagnoses was 79%. The main complication was stomal stenosis, 30%. CONCLUSIONS: These figures confirm that MACE is a useful operation. Improved patient selection, improvement in techniques, and increasing knowledge regarding continued management should lead to improving results across the country.

How to achieve a successful Malone antegrade continence enema.

BACKGROUND/PURPOSE: The Malone antegrade continence enema (MACE) has proved invaluable in the management of children with faecal incontinence. The authors have reviewed their experience in depth to try and ascertain why some MACEs fail. METHODS: The records of 31 children who had a MACE procedure between 1990 and 1995 were reviewed. Their preoperative bowel management, the operative procedure, the postoperative washout regime, and the outcome were assessed. Success was graded either as full, described as totally clean or minor rectal leakage on the night of the washout; partial, described as clean but significant stomal or rectal leakage, occasional major leaks, still wearing protection, and perceived improvement by the parents or child; or failure, described as regular soiling or constipation, no perceived improvement, and the procedure abandoned. There were 18 boys and 13 girls in the series. The mean age at survey was 8.4 years. RESULTS: Overall success rate was 19 of 31 (61%) with a mean follow-up of 3.25 years. The diagnosis and success rates were: anorectal anomaly 11 (success, 8 of 11, 73%), neuropathic bowel 11 (8 of 11, 73%), chronic constipation 8 (3 of 8, 38%).

Day-case ligation of patent ductus arteriosus in premature infants.

Over a 3-year period patent ductus arterious (PDA) ligation was performed on a day-case transfer basis on 45 premature infants. The overall survival rate was 93%. We would recommend this practice as an alternative to surgery in the neonatal intensive care unit.

Congenital mesoblastic nephroma, renin production, and hypertension.

Between 1964 and 1987, 12 patients with mesoblastic nephroma were treated. The BP was measured preoperatively in five patients, four of whom were hypertensive. Following nephrectomy, the BP returned to normal. Plasma renin levels were measured in one patient; they were markedly elevated pre-operatively, but returned to normal following tumour excision. Immunoreactive renin staining, using a polyclonal antibody to human renin, was performed in the 12 patients. Staining was positive in ten patients. The most intense staining was noted in the areas of cortex entrapped within the tumour, and in perivascular spaces not associated with entrapped cortex. These findings suggest that hypertension secondary to increased tumour associated renin production is a feature of congenital mesoblastic nephroma.

The functional sequelae of sacrococcygeal teratoma.

Twenty-seven patients with sacrococcygeal teratoma were followed for a mean period of 5 years (range, 2 to 12 years). Eleven (41%) had some form of functional impairment. Fecal and/or urinary incontinence was present in nine children and two had weakness of the lower limbs secondary to sciatic nerve palsy. Tumors with large intrapelvic extensions requiring an abdominoperineal approach for resection were associated with a higher incidence of functional sequelae (67%). It is recommended that patients with sacrococcygeal teratoma (particularly those with a presacral extension) be closely monitored for functional deficits so that early treatment can be instituted.

Exomphalos major, oesophageal atresia and tracheooesophageal fistula--a rare combination of major malformations.

Exomphalos major, oesophageal atresia and tracheooesophageal fistula are rare but well described birth defects. Both may be associated with other anomalies but rarely have been described as occurring together. We present a neonate born both premature and with these anomalies who presented major management difficulties.

Diagnosis of meconium peritonitis by orchidopexy.

Meconium peritonitis is an uncommon condition almost invariably associated with structural ileal obstruction or meconium ileus. Rarely meconium peritonitis may occur in an isolated form when the clinical course may be benign. We report a case where evidence of meconium peritonitis was only discovered during orchidopexy.

Oral lactose tolerance test in foals: technique and normal values.

Oral lactose tolerance tests were evaluated in 25 healthy foals (principals) assigned to 4 groups of approximately 1 week, 4 weeks, 8 weeks, and 12 weeks of age. Lactose monohydrate (1 g/kg of body weight [in a 20% water solution]) was administered via nasogastric tube after a 4-hour fast. Plasma glucose concentrations were monitored before dosing (0 minutes) and sequentially for 300 minutes. Six control foals were given a volume of water equivalent to the volume of lactose monohydrate administered to principal foals. After oral lactose loading, mean plasma glucose concentrations of all principal foals increased from 99.76 mg/dl at 0 minutes to 176.80 mg/dl by 90 minutes. Peak increases in plasma glucose concentrations were attained by 8% of the foals (2 foals) at 30 minutes, 76% (19 foals) at 60 minutes, and 16% (4 foals) at 90 minutes. The mean plasma glucose concentration increase of principal foals, regardless of age or time of peaking, was 77.04 mg/dl. There was no significant (P greater than 0.05) difference in fasting plasma glucose concentrations (0 minutes) among the 4 groups of principal foals or between principal and control foals; however, there was a significant (P less than 0.05) difference in peak glucose concentrations between 1-week-old and 12-week-old principal foals, with the older foals having the higher concentrations. Mean plasma glucose concentrations of control foals decreased from 79.67 mg/dl at 0 minutes to 55.17 mg/dl by 180 minutes. The mean peak decrease in plasma glucose concentrations of control foals, regardless of time of peaking, was 24.50 mg/dl.(ABSTRACT TRUNCATED AT 250 WORDS)

Endoscopic thoracic sympathectomy in the treatment of upper limb hyperhidrosis.

The technique of endoscopic sympathectomy is described and its value in the treatment of upper limb hyperhidrosis is reported in an initial series of 7 patients (13 sympathectomies). It is recommended as the treatment of choice for this condition.

Antenatal diagnosis of renal tract anomalies: has it increased the sum of human happiness?

When used as a screening procedure, ultrasound examination of the fetal urinary tract seldom leads to beneficial interventions. There is also a cost in terms of parental anxiety and unnecessary investigation and treatment. A formal screening programme would therefore be unjustified. However, screening of women for obstetric purposes will continue to reveal fetal abnormalities, and a strategy for dealing with these is needed. Antenatal treatments remain experimental; for most of the common conditions postnatal treatment has no urgency; and, in cases of minor abnormality detected by ultrasound, the best course may be to do nothing.

Intravesical botulinum type-A toxin (Dysport®) in the treatment of idiopathic detrusor overactivity in children.

OBJECTIVE: Botulinum type-A toxin is increasingly used for refractory idiopathic detrusor overactivity (IDO) in children. We reviewed our experience and sought to ascertain the influence of dose and functional bladder capacity on outcome. PATIENTS AND METHODS: Thirty patients, aged 6-16 years, with urodynamically proven IDO, had intravesical injections of 400-500 iu of Dysport(®). Outcome was assessed clinically at least 5 months after the injection. RESULTS: Data were available for 27 patients. Urinary frequency was improved in 10; nocturia was improved in 7. Urgency resolved in 10 patients and urge incontinence in 12 (44%). Complications reported were UTI (7), urinary retention (1) and bladder pain (1). The dose of Dysport(®) used was not significantly higher (14 iu/kg v 13 iu/kg) in patients dry at follow up than in those who remained wet (p = 0.45). Functional bladder capacity was not significantly different in patients dry after treatment (p = 0.82). CONCLUSION: This retrospective study demonstrates similar response to a single treatment with intravesical Botulinum type-A toxin to previous series. We did not demonstrate a correlation between dose or functional bladder capacity and resolution of incontinence. A multi-centre study is required to further investigate this promising treatment.

The anatomical assessment of disorders of sex development (DSD).

The assessment of abnormal anatomy in cases of DSD is important to aid diagnosis, understand the aetiology and severity of the condition, guide management and assess the outcomes of treatment. In this paper we present a systematic approach to this assessment which will provide a means by which the multiple disciplines who manage patients with these rare and complex conditions can communicate and thus improve overall care.